scholarly journals Mantle cell lymphoma of the prostate

2013 ◽  
Vol 3 (6) ◽  
pp. 94 ◽  
Author(s):  
Abuhjar Abdussalam ◽  
Ronald G. Gerridzen

Lymphoma of the prostate, either primary or secondary, is veryrare. We report the case of an 82-year-old man with symptoms ofbladder outlet obstruction presumably due to benign prostatehyperplasia (BPH). He underwent an uneventful transurethral resectionof the prostate. Pathological review of the resected tissuedemonstrated substantial infiltration by an atypical lymphoid infiltratepositive for CD20, BCL2, CD5, k light chain and cycline D1.Histology and immunoprofile were consistent with mantle celllymphoma.

2012 ◽  
Vol 160 (1) ◽  
pp. 63-69 ◽  
Author(s):  
Michelle Furtado ◽  
Nimish Shah ◽  
Alison Levoguer ◽  
Stephen Harding ◽  
Simon Rule

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3048-3048 ◽  
Author(s):  
Arshia Soleimani ◽  
Georges Tanios ◽  
Hana Safah ◽  
Nakhle S. Saba

Abstract Introduction: Mantle cell lymphoma (MCL) is an aggressive subtype of non-Hodgkin lymphoma (NHL) with a median survival of 3 to 5 years. MCL is characterized by the translocation t(11;14)(q13;32) which results in the overexpression of cyclin-D1 and ultimately an uninhibited G1/S cell cycle transition. MCL has a distinctive immunophenotype among B-cell NHL, characterized by a strong expression of the pan B-cell markers CD19 and CD20, absence of CD10 and CD23, overexpression of the anti-apoptotic protein BCL2, and aberrant expression of the T-cell marker CD5. However, up to 10% of MCL lack CD5 expression and remain ill-characterized. Whether this absence of CD5 expression impacts MCL biology and clinical course remains unknown. We performed a systematic review of all reported cases of CD5-negative MCL and analyzed their biological and clinical characteristics. Patients and methods: A systematic literature search was performed and included studies published from 1st June 1994 to 1st June 2016 in PubMed, Embase, and Web of Science. We used the key words CD5 negative, CD5-, mantle cell lymphoma, and MCL. Data was tabulated regarding the following 16 variables: t(11;14)(q13;q32), cyclin-D1, CD5, CD10, CD19, CD20, CD23, BCL2, BCL6, Ki-67 (higher or lower than 30%), IGHV mutation status (mutated IGHV >3% discordance with germline), male to female ratio, light chain expression (kappa or lambda), presence of a leukemic phase, stage, and overall survival (OS). Data was reported as percent and total number of cases with available data of each variable. Results and discussion: 68 out of 470 screened articles included CD5-negative MCL cases. After exclusion of duplicates, 50 sources (46 full articles and 4 abstracts) were included. Data of 222 cases of CD5-negative MCL were collected. To a degree, reviewed cases of CD-5 negative MCL shared similarities to classic MCL. Both sub-types had a median age at diagnosis of approximately 65 and presented as stage IV disease 70% of the time. Both subtypes overexpressed cyclin-D1 (100%, 222/222), possessed the t(11;14)(q13;q32) (93%, 49/53: 49 of 53 cases with available data on the translocation), were CD-19 positive (100%,18/18), CD20-positive (100%, 58/58), BCL2-positive (97%, 34/35), CD10-negative (93%, 42/45), and CD23-negative (95%,56/59). Presence or absence of leukemic phase was reported in 16 cases, 8 of those were positive for circulating MCL cells (50%). The proliferation marker Ki-67 was reported in 22 cases, 7 (32%) of which were >30%. CD5-negative MCL deviated from the classic MCL presentation in a number of clinical and biological variables. Most importantly, the median OS of 47 cases with available follow-up was greater than 16 years (Figure 1), which compared very favorably to the historic survival of 3 to 5 years in classic MCL. Interestingly, the number of affected males was less than expected in the CD5-negative group. Male-to-female ratio was 2:1 (74 males and 36 females of 110 cases with available gender data), which is lower than the expected 3:1 ratio. From a biological standpoint, the CD5-negative MCL cohort had more kappa than lambda-restricted cases (62%, 13 of 21 available light chain data), and more mutated than unmutated IGHV (61% mutated, 11 of 18 cases with available IGHV data). Classically, lambda-restricted MCL and unmutated IGHV are more commonly seen and were associated with worse clinical outcomes. In addition, we observed BCL6 expression in 23% of CD5-negative MCL cases, which is higher than expected (6 of 26 cases with BCL6 available data). Conclusions: To our knowledge this is the most comprehensive review of CD5-negative MCL. Lack of CD5 expression was associated with important clinical and biological differences compared to classic MCL. The higher survival observed in our analysis suggest that CD5 can potentially be incorporated in identifying a subset of a more indolent MCL that might benefit from a watch and wait approach. Currently, treatment in MCL can be differed in a subset of patients with an indolent presentation characterized by leukemic-only disease with absence of lymphadenopathy, mutated IGHV, and lack of SOX11 expression. SOX11 data was not available in our cohort, and only 18 patients had available IGHV mutation data. While the exact role of CD5 in MCL remains unknown, our findings highlight the need for a deeper investigation of CD5-negative MCL at the genetic, phenotypic, and clinical levels. Disclosures No relevant conflicts of interest to declare.


1999 ◽  
Vol 36 (1-2) ◽  
pp. 147-150 ◽  
Author(s):  
Francesco Bertoni ◽  
Emanuele Zucca ◽  
Davide Genini ◽  
Gianni Cazzaniga ◽  
Enrico Roggero ◽  
...  

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S127-S127
Author(s):  
K M Erickson ◽  
D Lynch

Abstract Casestudy: Chronic lymphocytic leukemia (CLL) accounts for about 30% of all lymphoid neoplasms and is the most common adult blood cancer in the Western world. Mantle cell lymphoma (MCL) accounts for only about 6% of all B-cell lymphomas in Western countries. MCL and CLL are both CD5 positive B-cell lymphoproliferative disorders. It is necessary to distinguish these two entities as MCL is a more aggressive disease, and requires specific treatment. MCL and CLL can occur in one patient at the same time and is often termed a composite lymphoma. We present an 84-year-old female with a history of endometrial cancer who was found to have splenomegaly and lymphadenopathy. Flow cytometry was performed upon her peripheral blood specimen which demonstrated two distinct populations of abnormal light chain restricted B-cell populations. One population demonstrated kappa light chain restriction and was positive for CD45, CD19, CD20, CD5, CD38, FMC-7, and CD22, representing MCL. The other population showed dim lambda light chain restriction that was also positive for CD45, CD19, dim CD20, CD5, and CD23, representing CLL. FISH studies demonstrated t(11;14), and four common deletions or chromosome aneuploidy associated with CLL. These findings confirmed the dual populations of CLL and MCL. This is an interesting case because it is a very rare combination with only a few cases having been reported with two distinct cell populations in one patient at the same time.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4814-4814
Author(s):  
John Zhang ◽  
David Chin ◽  
Adam Anthony ◽  
Heather Bolton ◽  
Cheri Phillips ◽  
...  

Abstract The differential diagnoses of CD5 positive B-cell lymphoproliferative disorders mainly include chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma. Occasionally large cell and marginal zone lymphomas may also be CD5 positive. An accurate diagnosis effects patient management. The classical immunophenotype for chronic lymphocytic leukemia/small lymphocytic lymphoma is CD19/CD5/CD23 positive FMC-7 negative cells with dim CD20 and dim light chain expressions, while mantle cell lymphoma is CD19/CD5/FMC-7 positive with bright CD20 and bright light chain expressions. The diagnosis of mantle cell lymphoma is usually confirmed by either immunostain for cyclin D1 or FISH study for t(11;14). In reality, immunostaining for cyclin D1 can be difficult and may show variable results in different laboratories and FISH study may not be readily available. Generally, when it comes to the diagnosis of lymphoma, immunohistochemical positivity of both CD5 and CD23 is almost pathognomic for chronic lymphocytic leukemia/small lymphocytic lymphoma if no fresh tissue is saved for flow cytometry analysis. Flow cytometry analysis of 44 FISH-confirmed mantle cell lymphomas was reviewed in our lab. Among these, 37 showed the classical immunophenotype of mantle cell lymphoma. However, 7 cases (16%) were positive for both CD5 and CD23. The expression of CD23 varied from dim to bright. When compared to typical CLL, they showed FMC-7 expression and brighter than dim light chain expression. In one case, the light chain expression was dim. In conclusion, CD23 expression which was thought to be a specific marker for CLL/SLL may also be seen with mantle cell lymphoma. Although FMC-7 expression is seen in all CD23 positive mantle cell lymphomas, bright light chain expression is not universal. We recommend that FISH or immunohistochemical studies for cyclin D1 be performed on CD5/CD19 clonal B cell proliferations with CD23 expression if morphology or immunophenotype is atypical for CLL/SLL.


2016 ◽  
Vol 177 (5) ◽  
pp. 816-818
Author(s):  
Carolina Villegas Da Ros ◽  
Mariano Linares Garcia ◽  
Sebastian Ortiz Zuluaga ◽  
Karla Javier Gonzalez ◽  
Sofia Costa ◽  
...  

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S23-S23
Author(s):  
K M Erickson ◽  
D Lynch

Abstract Casestudy: Chronic lymphocytic leukemia (CLL) accounts for about 30% of all lymphoid neoplasms and is the most common adult blood cancer in the Western world. Mantle cell lymphoma (MCL) accounts for only about 6% of all B-cell lymphomas in Western countries. MCL and CLL are both CD5 positive B-cell lymphoproliferative disorders. It is necessary to distinguish these two entities as MCL is a more aggressive disease, and requires specific treatment. MCL and CLL can occur in one patient at the same time and is often termed a composite lymphoma. We present an 84-year-old female with a history of endometrial cancer who was found to have splenomegaly and lymphadenopathy. Flow cytometry was performed upon her peripheral blood specimen which demonstrated two distinct populations of abnormal light chain restricted B-cell populations. One population demonstrated kappa light chain restriction and was positive for CD45, CD19, CD20, CD5, CD38, FMC-7, and CD22, representing MCL. The other population showed dim lambda light chain restriction that was also positive for CD45, CD19, dim CD20, CD5, and CD23, representing CLL. FISH studies demonstrated t(11;14), and four common deletions or chromosome aneuploidy associated with CLL. These findings confirmed the dual populations of CLL and MCL. This is an interesting case because it is a very rare combination with only a few cases having been reported with two distinct cell populations in one patient at the same time.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 398-398
Author(s):  
Harvey A Greisman ◽  
Zhengfei Lu ◽  
Albert G Tsai ◽  
Timothy C. Greiner ◽  
Hye Son Yi ◽  
...  

Abstract Abstract 398 Most t(11;14)(q13;q32) breakpoints in mantle cell lymphoma (MCL) are scattered across a large genomic region centromeric to the CCND1 gene on chromosome 11q13. However, few t(11;14) breakpoints outside the major translocation cluster (MTC) have been sequenced. We report our analysis of 56 IGH-CCND1 fusion sequences from 32 non-MTC cases of MCL. Our analysis reveals remarkable breakpoint diversity at both CCND1 and IGH loci. The CCND1 breakpoints are located from 2 kb to 331 kb from the CCND1 gene, including 12 breakpoints (38%) in the 220kb region centromeric to the MTC, which is itself located 110 kb centromeric to the CCND1 gene. Twenty-one cases had a JH coding end breakpoint on the der(14) chromosome and a DH coding end breakpoint on the der(11). Two cases had der(14) and der(11) breakpoints derived from the coding and signal ends of the same JH or DH gene segment; one case had a JH/DH breakpoint on the der(14) and a VH breakpoint on the der(11); and one case had a breakpoint in the JH region located >100 bases from the nearest RSS motif, suggesting a RAG-independent break at IGH. No der(11) breakpoint could be amplified in eight cases. In sixteen cases (50%), one or both CCND1 breakpoints were within 4 bases of a CpG dinucleotide, a feature characteristic of translocation breakpoints in many human B cell lymphomas. Three CpG-associated “microclusters” were identified, i.e. breakpoints in two different tumors located at or near the same CpG site. Breakpoints in the sixteen (50%) “non-CpG” cases showed a significant association with AID hotspot motifs at the CCND1 locus. In addition, the non-CpG breakpoints were more likely to: 1) be located telomeric to the MTC, 2) involve 3' JH and 5' DH gene segments, and 3) be associated with immunoglobulin lambda light chain restriction; features that suggest occurrence at a late stage of pre-B cell maturation. In contrast, the CpG-associated breakpoints were: 1) more likely to be centromeric to the MTC, 2) not biased in their JH or DH segment usage, and 3) associated with kappa light chain restriction; features suggesting occurrence in an earlier pre-B cell or pro-B cell. Our results implicate AID in chromosomal breakage at both CpG and non-CpG sites within the CCND1 locus and suggest that AID and RAG collude to generate the chromosomal breaks underlying the t(11;14). Our findings also suggest that IGH-CCND1 rearrangements can occur at different stages of pre-B cell maturation. This study provides novel insights into the mechanism and developmental timing of the t(11;14) in human MCL, features that are likely to be relevant to a broad range of human lymphomas. Disclosures: Greisman: Signature Genomics, LLC: Patents & Royalties. Yi:Signature Genomics, LLC: Patents & Royalties.


Blood ◽  
2021 ◽  
Author(s):  
Lorenz Thurner ◽  
Natalie Fadle ◽  
Jörg Thomas Bittenbring ◽  
Evi Regitz ◽  
Rita Schuck ◽  
...  

Recently, we identified LDL receptor related protein associated protein 1 (LRPAP1) as frequent autoantigen of recombinant B-cell receptors (BCRs) of mantle cell lymphoma (MCL). This autoantigen induced proliferation in two cell lines with BCR-reactivity against LRPAP1. Of interest, high-titered and light-chain-restricted LRPAP1-autoantibodies were detected in 8 of 28 patients with MCL. In the present study, LRPAP1-autoantibodies in sera of patients treated within the Younger and Elderly trials of the European MCL Network were analyzed regarding frequency, association with disease characteristics and prognostic impact. LRPAP1-autoantibodies were detected in 41 (13 %) of 312 evaluable patients with MCL. These LRPAP1-autoantibodies belonged predominantly to the IgG class (IgG: 37; IgM: 4, IgG-subclasses: IgG1: 25, IgG2: 7, IgG3: 4 and IgG4: 1) and were clonally light-chain-restricted (27 with kappa light-chains, 14 patients with lambda light-chains). Titers ranged between 1:400 up to 1:3200. The presence of LRPAP1-autoantibodies was not significantly associated with any baseline clinical characteristic. However, the presence of LRPAP1-autoantibodies was associated with a superior 5-year probability for failure-free survival (FFS) of 70% (95% CI: 57%-87%) vs. 51% (95% CI: 44%-58%) p:0.0052; and for overall survival (OS) of 93% (95% CI: 85%-100%) vs. 68% (95% CI 62%-74%), p=0.0142. LRPAP1-seronegative patients had a MIPI-adjusted HR for FFS of 2.1 (95% CI 1.2-3.6, p=0.0083) and for OS of 2.1 (95% CI 1.07-4.2, p=0.032). LRPAP1-autoantibodies were frequently detected in serum samples of a large cohort of MCL patients treated within prospective multicenter clinical trials. Our results suggest better outcomes for LRPAP1-autoantibody seropositive patients.


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